Department of Neurology, Columbia University, New York, NY 10032, USA.
Neuron. 2011 Jun 23;70(6):1033-53. doi: 10.1016/j.neuron.2011.06.003.
Adult-onset neurodegenerative disorders are disabling and often fatal diseases of the nervous system whose underlying mechanisms of cell death remain unknown. Defects in mitochondrial respiration had previously been proposed to contribute to the occurrence of many, if not all, of the most common neurodegenerative disorders. However, the discovery of genes mutated in hereditary forms of these enigmatic diseases has additionally suggested defects in mitochondrial dynamics. Such disturbances can lead to changes in mitochondrial trafficking, in interorganellar communication, and in mitochondrial quality control. These new mechanisms by which mitochondria may also be linked to neurodegeneration will likely have far-reaching implications for our understanding of the pathophysiology and treatment of adult-onset neurodegenerative disorders.
成人发病的神经退行性疾病是使人丧失能力且往往致命的神经系统疾病,其细胞死亡的潜在机制仍不清楚。先前有人提出,线粒体呼吸缺陷可能导致许多(如果不是全部的话)最常见的神经退行性疾病的发生。然而,在遗传性形式的这些神秘疾病的基因突变的发现,也提示了线粒体动力学的缺陷。这种干扰会导致线粒体运输、细胞器间通讯和线粒体质量控制的变化。线粒体与神经退行性变相关的这些新机制很可能对我们理解成人发病的神经退行性疾病的病理生理学和治疗具有深远的影响。